外科治疗胸腺瘤合并单纯红细胞再生障碍性贫血(附5例报告及国内文献复习)

Experience of surgical management of thymoma associated with pure red cell aplasia: five cases report and review of Chinese literatures

目的 探讨国人胸腺瘤合并单纯红细胞再生障碍性贫血 (PRCA)的临床特征及其外科治疗效果。方法 总结 1980年至 1997年间 5例胸腺瘤合并PRCA病人接受胸腺瘤手术治疗的资料 ,并通过中国生物医学文献数据库光盘进行文献检索 ,对中国大陆已报道的这类病例进行文献复习和讨论。结果 本组胸腺瘤合并PRCA者占同期胸腺瘤病人的 8 3% (5 6 0例 )。全部均经胸骨正中切口切除胸腺瘤及胸腺组织和前纵隔脂肪。术后 45d死亡 1例 ,PRCA复发 2例 ,长期生存 2例。迄今为止 ,国内共检索出此类病人 2 1例 ,术后早期PRCA情况明显改善 ,按术后随访满 2年 ,且无PRCA复发征象作为手术有效标准 ,有效率为 38 5 %。结论 胸腺瘤合并PRCA是一种少见疾病 ,其预后取决于PRCA的缓解程度 ,胸腺瘤切除术对治疗PRCA是有效的 ,外科治疗是本病的首选治疗手段 ,也是其他综合性治疗方法的前提和基础。

Objective: To better understand the clinical features of thymoma associated with pure red cell aplasia(PRCA) and to evaluate outcome of surgical treatment. Methods: From 1980 to 1997, 5 cases of thymoma associated with PRCA, accounting for 8 3% of total thymomas treated in the same period, underwent thymomectomy. The clinical data was analysed and the Chinese literature was reviewed. Results: The early results of thymomectomy were excellent. The hemoglobin concentration and reticulocyte counts returned to normal level. One patient died of pulmonary infection on the 45th postoperative day. Recurrence of PRCA were found in 2 patients 3-4 months after operation. Long term survival was achieved in 2 cases, one survived over 17 years and another over 3 years without recurrence of PRCA. Twenty one operative cases of thymoma with PRCA were reported in Chinese literature, the effectiveness of thymomectomy is 38 5%. Conclusion: Thymoma associated with PRCA is a rare autoimmune disease. Its outcome depends on the degree of PRCA remission. Surgery for thymoma is still the top priority in the selection of treatment.